BB LECT JUNE 17,2015
Le (a-b+) 72 55
SUMMARY OF CLINICALLY SIGNIFICANT BLOOD
GROUP
CLINICAL BLOOD GROUP SYSTEM
SIGNIFICANCE ALLOANTIBODIES
Clinical significant ABO,Rh,Kell,Kidd,Duffy,S,s
and U, Lutheran (Lub)
Usually clinically I, Lewis,M,N,P1,Lutheran
insignificant (Lua)
LEWIS SYSTEM (Le)
 The only blood group not manufactured
by rbc, but secreted by tissue cells
secreted into body fluids
 Produce specific enzyme
glycosyltransferase & I-
fucosyltramsferase that adds L-fucose
to precursor substance
 Located on chromosome 1
 Inheritance of the Le gene acts in
competition with ABO genes adding L-
fucose to the GlcNAc sugar of the
common precursor structure
manufactured by tissue cells (Lea soluble
antigen)
 The frequency of Lewis phenotypes
parallel the frequency of the secretor
gene
 Le is distantly linked to Se and H genes
since they are located on the same
chromosome
 They are glycoprotein in nature in
secretions (80% carbohydrates &15%
amino acids)
 They are glycolipid in plasma
BASIC CONCEPTS OF LEWIS
PHENOTYPES
Phenotype Whites (%) Blacks (%)
Le(a+b-) 22 22
Le(a-b-) 6 23
Lw(a+b+) 0 -
Lewis (a+b-) Phenotype/Nonsecretors
 All are nonsecretors of ABH
substance results from the
transfer of fucose to type 1
chain by the action pf enzyme
L-fucosyl transferase
Lewis (a-b+) secretors
 Result og genetic interaction of
Lele and Sese genes
 Leb antigen represents the
product of genetic interaction
between Le (FUT3) and Se (FUT2)
genes
 The addition of another L-fucose
added to subterminal the Leb
Lewis (a-b-): Secretors and non-
secretors
 Lack of Lewis antigen on
RBC caused by point
mutation in the Le gene
 The mutation give rise to a
non-functional or partially
active Lewis transferase
(Lew) causing the negative
expression of the Lewis
antisera
 Main substance found in
the secretions of Le (a-b-)
individuals depend on the
secretor status and ABO
genotype of the person
 Le (a-b-) nonsecretors
express type1 precursor, Le
(a-b-) secretors express H
 type1 substances plus ABH
antigens associated with
the related ABO genes
Changes in Lewis phenotype (Le a-b-)
 This maybe due to physiologic changes
in the composition of blood that affect
the distribution of Lewis glycolipid
between plasma and the RBCs
 Large increase in the ratio of plasma
lipoprotein to RBC mass that occurs
during pregnancy
 Seen also in patients with cancer
alcoholic cirrhosis, viral & parasitic
infections
Lewis antibodies
 Frequently detected in antibody
screening procedures
 Produced by Le (a-b-) persons
 Naturally occurring
 IgM and don’t cross the placenta
 They active complement
 May cause in vivo hemolysis of red cell
 Sometimes reacts at 37C and coombs
phase more weakly than a room
temperature
 Enhanced by enzymes
 Readily neutralized by Lewis blood
group substance
Anti-Lea
 Most commonly encountered Le
antibody
 IgM in nature
 Reactivity is enhanced by enzyme
treated rbc
 Frequently detected with saline-
suspended cells at room temperature
 May react at 37C and coombs phase
and therefore associated with
hemolytic transfusion reaction
 Easily neutralized with plasma or saliva
that contains Lea
Anti- Leb
 IgM agglutinin, don’t fix complement
 Produced by Le(a-b-) individual
 Neutralized by plasma or saliva
containing Leb
Secretor genes
 Individuals who inherits Se allele
and expresses soluble forms of H
antigens in secretions
 Nonsecretors- individual who
inherits the genotype sese and does
not express soluble H substance in
secretions
 Secretor studies is helpful in
identifying a subgroups of A or B
antigens
 2 allelic forms: Se and se
 Soluble antigens are found in saliva,
urine, tears,amniotic fluid,
breastmilk,exudates and digestive
fluids
 An individual with sese genotype-
nonsecretor
 Se allele is considered amorph, thus
homozygous allele does not convert
glycoprotein precursor to soluble H
substance
 Genes inherited Antigen  43 antigens are included in the system
expression  Low in incidence and discovered in
AB HH Sese A,BH,both in cases of HDN or incompatible
RBC and Saliva
crossmatch
AB,HH,sese ABH in RBC,
None in saliva SYSTE ANTIGEN CLAS COMMEN
M S S TS
OO,HH,sese H(RBC)none
(saliva) MNS M IgM Antigens
M and N
MNSs Blood System
show
 4 important antigens (more exist): dosage
N IgM S-s are
M
also U-
N negative
S S IgG
S
U(always present when S&s are Frequency of MNSs antigens
inherited) Phenotypes Blacks(%) Whites
 M & N located on Glycophorin A (%)
 S & s and U located on Glycophorin B M+ 74 78
 Remember: Glycophorin is a protein N+ 75 72
S+ 30.5 55
that carries many RBC antigens
s+ 94 89
U+ 99 99.9

MNSs antigens
 All show dosage
 M & N give a stronger reaction when
homozygous,(M+N-) or (M-N+)
 Weaker reactions occur when in the
heterozygous state (M+N+)
 Antigens are destroyed by enzymes (i.e.
ficin,papain)
MNSs blood group system
S and s antigens
 Located on GPB
 Differentiated by amino acids
(S- methionine, s- thereonine)
 Well- developed at birth
 Less easily degraded by
enzymes due to less enzyme
accessibility
 Not found on platelets,
lymphocytes, monocytes, &
granulocytes
Anti –M&Anti-N
 Naturally occurring saline agglutinins
that react below 37c(cold reacting abs)
 IgG& don’t bind complement
 Don’t react with enzyme treated RBC’s
 Common in children than adults & burn
patients
  Anti-N seen in renal patients, and
associated in chilled transplanted
kidney
I blood group system & I antigen
Enhancing Anti- M reactivity
 Increasing the serum cell ratio or
incubation time or both
 Decreasing incubation time or by
adding potentiating medium such as
albumin. Low ionic strength
solution(LISS) or polyethylene glycol
(PEG)
 Pantigen can be agglutinated by
hydatid cyts fluid & pigeon eggs
 I stands for individuality discovered
by Weiner in 1956 found in patients
with hemolytic anemia
 I and i are formed from glycosyl
transferase and have reciprocal
relationship
I blood group

Anti- S and Anti-s & anti- U Syste Antigen Clas Comments

m s s
 IgG & reactive at 37c and the I I IgM Frequently
antiglobulin test phase found as cold
autoantibodie
 If present, incubate test at room
s, I is negative
temperature and performing the on cord cells
antiglobulin test without incubating 37c I IgM
 May not react with enzyme treated
RBCs
 80% of whites and 95% of blacks have Anti-I
the P1 phenotypes: anti P1,P2,p,P1k,p2k  Common antibody that can be
 Rare p phenotype refers to the found in all sera
absence of P antigens and they  Weak,naturally occurring,
make potent anti-PP1 Pk saline- reactive IgM
 Antibodies to P antigens are most autoagglutinin usually detected
commonly seen in P2 phenotype only at 4C.
individuals. IgM and anti P1 that are  Pathogenic anti-I is typically a
reactive at 4C & not clinically strong cold autoagglutinin that
significant demonstrates high titer
reactivity at 4C and reacts over
 P antigens are the target antibodies
a wide thermal range up to 30-
in paroxysmal cold hemoglobinuria
32C
(PCH). Not routine detected in
 Potent cold autoantibodies can
antibody screen but with Donath- mask clinically significant
Lansdteiner test underlying alloantibodies and
 The P antigen serves as the receptor complicate pretransfusion
for parvovirus b19 testing
  Patients with M.pneumoniae  The mcleod syndrome includes the
infections may develop strong clinical manifestation of abnormal rbc
cold agglutinins with autoanti-I morphology and compensated
specificity hemolytic anemia and neurologic and
 Anti-I is a rare IgM agglutinins muscular abnormalities
that reacts optimally at 4C,
The Duffy blood group system
potent examples may be
associated with infections  Fya and Fyb antigens are destroyed
mononucleosis by enzymes and ZZAP; they are well
Kell Blood Group System developed at birth. The Fy(a-b-)
phenotype is prevalent in blacks but
 The blood group antigens are well virtually nonexistent in whites.
developed at birth and are not  Fu(a-b-) RBCs were shown to resist
destroyed by enzymes infection by the malaria organisms
 The kell blood group antigens are P.Knowlesi and P.vivax.
destroyed by DTT, ZZAP and glycine
Duffy Blood group
acid-EDTA
 Excluding ABO, the K antigen is rated SYSTE ANTIGEN CLAS COMMEN
second only to D antigen in M S S S
immunogenicity Duffy Fya IgG Fy(a-b-) is
protective
Kell blood group against
malaria
SYSTEM ANTIGENS CLASS COMMENTS Fyb IgG
Kell K IgG Antigens in  Anti Fya and Anti Fyb are usually IgG
the Kell
antibodies and react optimally at the
system is
antoglobulin phase of testing: both
destroyed
by DTT antibodies have been implicated in
k IgG delayed hemolytic transfusion reactions
Kpa IgG and HDN.
b
Kp IgG
Jsa IgG The Kidd blood group system
Jsb IgG  Anti-Jka and Anti Jkb may demonstrate
 The k antigen is high incidence antigen dosage; are often weal, and are we
 Anti K is usually an IgG antibody found in combination with other
reactive in the AHG phase and is made antibodies; both are typically IgG and
in response to pregnancy or transfusion antiglobulin-reactive.
reactions & HDN
 Kidd system antibodies may bind
 Mcleod phenotype, affecting only males complement and are made in response
,is decreased kell system antigen to foreign RBC exposure during
expression pregnancy or transfusion.
 SYSTE ANTIGEN CLAS COMMEN COLD ANTIBODIES (IgM)
M S S TS
Kidd Jka IgG Associated  Anti-Lea
w/  Anti-Leb
delayed  Anti-I
transfusio
 Anti-P1
n
reactiona  Anti-M
Jkb IgG  Anti-A,-B,-H
 Kidd system antibodies are  Anti-N
common cause of delayed LIiPMABHN (naturally occurring)
hemolytic transfusion reactions.
WARM ANTIBODIES (IgG)
 Kidd system antibody reactivity
is enhanced with enzymes, LISS,  Rh antibodies
and PEG.  Kell
 Duffy
Lutheran blood Group System
 Kidd
 Lua and Lub antigens produced by allelic  S,s
codominant genes; they are poorly
developed at birth. REMEMBER ENZYME ACTIVITY:
 Anti- Lua may be naturally occuruing Papain,bromelin,ficin,trypsin
saline agglutin that reacts optimally at
room temp. ENHANCED BY DESTROYED BY
 Anti Lub is an IgG ab reactive at the AHG ENZYMES ENZYMES
phase; usually produced in response to Kidd,Rh,Lewis,I,P Fya and Fyb,M,N,S,s
foreign RBC exposure during pregnancy
or transfusion REMENBERING DOSAGE:
 The Lu(a-b-) phenotype is race and may
result from three different genetic Kidds and Duffy the (Rh) eat lots of M&Ns
backgrounds
Jka,Jkb- kidd
System Antigens Class Comments
Fya,Fyb-duffy
Lutheran Lua IgM May
exhibit
C,c,E,e(no D)- Rh
mix field
reactions M,N,S,s-MNSs
Lub IgM